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Table 1 Baseline Patient Demographic and Cardiovascular Imaging Data

From: Prospective cardiovascular magnetic resonance imaging in adults with Alström syndrome: silent progression of diffuse interstitial fibrosis

Factor

N

Statistic

Demographics

 Age at first scan (years)

30

21 (19–33)

 Male Gender (%)

30

19 (63%)

 White British (%)

30

20 (67%)

 BMI (kg/m2)

30

29 (26–32)

 Systolic BP (mmHg)

30

130 ± 19

 Diastolic BP (mmHg)

30

82 ± 12

 Heart rate (beats/min)

30

86 ± 17

CMR

 T1 Basal (ms)

30

937 (913–1030)

 T1 SAX Basal ECV (%)

30

27 (22–33)

 LVEDVi (mL/m2)

30

58 ± 12

 LVEF (%)

30

64 ± 9

 LV mass index (g/m2)

30

55 (51–61)

 LAV index (mL/m2)

30

34 (25–43)

 GLS (%)

30

16 ± 3

Echocardiogram

 E/A ratio

29

1.5 (1.3–1.6)

 Average E/e’ ratio

30

7.4 ± 2.1

 e’ ave. (cm/s)

27

9.1 (7.7–11.0)

Blood markers

 Haemoglobin (g/dL)

30

14.0 ± 2.3

 HbA1c (mmol/mol)

29

49 (39–71)

 C-peptide (pmol/L)

30

3136 (1516–4113)

 Cholesterol (mmol/L)

30

4.8 ± 1.3

 Triglycerides (mmol/L)

30

2.5 (1.7–4.2)

 NT-proBNP (ng/L)

30

51 (25–93)

 eGFR (mL/min/1.73 m2)

30

108 (62–123)

Comorbidities

 Hypertension

30

17 (57%)

 Dyslipidaemia

30

18 (60%)

 Infantile cardiomyopathy

30

12 (40%)

 Diabetes

30

18 (60%)

 CKD

30

18 (60%)

  1. BMI indicates body mass index, BP blood pressure, CMR cardiovascular magnetic resonance, E/A ratio mitral early filling (E)/atrial filling (A) ratio, E/e’ mitral early filling (E)/early myocardial relaxation velocity (e’), ECV extracellular volume, eGFR estimated glomerular filtration rate, GLS global longitudinal strain, HbA1c glycated haemoglobin A1c, IQR interquartile range, LAV left atrial volume, LV left ventricular, LVEDVi left ventricular end-diastolic volume indexed to body surface area, LVEF left ventricular ejection fraction, LVESVi left ventricular end-systolic volume indexed, NT-proBNP serum Nterminal pro B natriuretic peptide, RV right ventricle, SAX short axis, CKD chronic kidney disease
  2. Data are reported as N (%), mean ± SD, or median (IQR), as applicable